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Major congenital heart diseases (CHD) often demand intervention in the neonatal period. Prenatal diagnosis may improve mortality by eliminating the diagnostic delay; however, there is controversy concerning its true effect. We aimed to evaluate the effect of general prenatal screening on prognosis by comparing a period without general prenatal screening to a period with general prenatal screening. We conducted a nationwide retrospective study including live born children and terminated fetuses diagnosed with major CHD. Prenatal screening was recommended only in high risk pregnancies between 1996 and 2004, whereas general prenatal screening was recommended between 2005 and 2013. We assessed the influence of general prenatal screening on all-cause mortality, cardiac death, preoperative and postoperative 30-day mortality and complication rate. 1-year mortality decreased over both periods, but the decrease was greater in the screening period (Odds ratio 0.92 (CI 0.83–1.00), p = 0.047). Prenatal detection of major CHD was associated with cardiac death in the period without general screening (Hazard Ratio 2.40 (CI 1.72–3.33), p < 0.001), whereas there was no significant association once general screening was implemented. Similarly, the association between prenatal diagnosis and pre- and postoperative mortality found in the period without general screening was insignificant after the implementation of general screening. Mortality in major CHD decreased throughout the study, especially in the period with general prenatal screening. However, comparing a prenatally diagnosed group with a postnatally diagnosed group is vulnerable to selection bias and proper interpretation is difficult. • Survival of major congenital heart disease is improving • The occurrence of complications is increasing, presumably due to improved survival • Prenatal diagnosis of major congenital heart disease is no longer associated with increased cardiac death. • Great care must be taken when comparing prenatally diagnosed congenital heart disease with postnatally diagnosed congenital heart disease [ABSTRACT FROM AUTHOR]

Background The ductus arteriosus (DA) is one of the most important vessels of the fetal circulation. A variety of fetal congenital heart disease (CHD) may greatly influence the structure and flow patterns of the DA. This study was to investigate the structural characteristics of the DA and its value in the diagnosis of fetal CHD using fetal echocardiography combined with cardiovascular casting technology. Methods Twenty-six cases of a normal fetus and 20 cases of a fetus with CHD (10 cases of right-sided obstructive CHD and 10 cases of left-sided obstructive CHD) were enrolled in this study. The three-vessel view and the long axis view of the DA arch were chosen to observe the fetal DA. The diameters of the ascending aorta (AO) and the pulmonary artery trunk (PA) were measured on the left ventricular outflow tract view and the three-vessel view separately, and AO/DA and PA/DA values were calculated separately. The flow direction of the DA was recorded, and the blood flow peak velocity was measured using color and spectral Doppler technology. Cardiovascular cast specimens were made for fetuses secured from induced labor to facilitate further observations of the true form and connections of the DA. At the same time, heart and great vessel deformities were also recorded through careful observation of the cardiovascular cast. Results The following DA anomalies were observed: ① abnormal diameter (6 cases of stenosis and 9 cases of dilatation); ② abnormal blood flow direction (reverse flow in 5 cases); ③ abnormal blood flow speed (12 cases); ④ abnormal connection site (right-sided DA in 2 cases and a DA connection between the left pulmonary artery and the left subclavian artery in one case); and ⑤ absence of the DA in 2 cases. Compared with the control group, the DA diameter in the right-sided obstructive group was obviously narrowed; by contrast, the diameter of the DA in the left-sided obstructive group was obviously dilated. These differences were statistically significant ( p < 0.05). Compared with the control group, the AO/DA values in the right- and left-sided obstructive groups were significantly increased and decreased, respectively. Finally, relative to the control group, the PA/DA values of the right- and left-sided obstructive groups were significantly reduced. Conclusions DA enlargement was often associated with left-sided obstructive CHD, whereas a small DA and reverse blood flow often indicated the presence of right-sided obstructive CHD. DA connections can manifest with multiple anatomical variations. During fetal echocardiography, evaluation of the DA is very important; such analyses may help search for associated cardiac defects whenever a diagnosis of CHD is made. [ABSTRACT FROM AUTHOR]

Background Quantitative analysis of mitral valve morphology with three-dimensional (3D) transesophageal echocardiography (TEE) provides anatomic information that can assist clinical decision-making. However, routine use of mitral valve quantification has been hindered by tedious workflow and high operator-dependence. The purpose of this paper was to evaluate the feasibility, accuracy and efficiency of a novel computer-learning algorithm using anatomical intelligence in ultrasound (AIUS) to automatically detect and quantitatively assess the mitral valve anatomy. Methods A novice operator used AIUS to quantitatively assess mitral valve anatomy on the 3D TEE images of 55 patients (33 with mitral valve prolapse, 11 with functional mitral regurgitation, and 11 normal valves). The results were compared to that of manual mitral valve quantification by an experienced 3D echocardiographer and, in the 24 patients who underwent mitral valve repair, the surgical findings. Time consumption and reproducibility of AIUS were compared to the manual method. Results AIUS mitral valve quantification was feasible in 52 patients (95%). There were excellent agreements between AIUS and expert manual quantification for all mitral valve anatomic parameters (r = 0.85–0.99, p < 0.05). AIUS accurately classified surgically defined location of prolapse in 139 of 144 segments analyzed (97%). AIUS improved the intra- [intraclass-correlation coefficient (ICC) = 0.91–0.99] and inter-observer (ICC = 0.86–0.98) variability of novice users, surpassing the manual approach (intra-observer ICC = 0.32–0.95; inter-observer ICC = 0.45–0.93), yet requiring significantly less time (144 ± 24 s vs. 770 ± 89 s, p < 0.0001). Conclusion Anatomic intelligence in 3D TEE image can provide accurate, reproducible, and rapid quantification of the mitral valve anatomy. [ABSTRACT FROM AUTHOR]

Abstract: Background: The incidence of children born with functional univentricular heart (UVH) and their prognosis presumably changed substantially in recent years. This is due to introduction of fetal echocardiography and potential termination of pregnancy (TOP) when UVH is diagnosed (UVH TOP), and to improvements in treatment. We aimed to explore changes in incidence, to estimate changes in survival, and to describe predictors of mortality in UVH patients. Methods: Using a population-based design we identified all UVH cases in Denmark from 1977 to 2009. Results: 703 UVH live births and 106 UVH TOP were identified. A dramatic decrease in birth incidence of UVH patients and a corresponding increase in UVH TOP was observed in recent years. Mean incidence rate of UVH (live births and UVH TOP) was 0.39 per 1000 births. In adjusted analysis survival improved significantly from birth era 1977–1989 to 1990–1999 (HR 2.65, 95% confidence interval (CI), 2.06–3.42) but not significantly from 1990–1999 to 2000–2009 (HR 0.77, 95% CI, 0.57–1.05). In the birth era 2000–2009, the lowest five-year survival was seen with hypoplastic left heart syndrome (HLHS) (18.8%), whereas the best survival was seen with tricuspid atresia (79.8%). Adjusted risk of death was 7.3 times higher in the HLHS group compared to the tricuspid atresia group (95% CI, 3.94–13.47). Conclusions: This study demonstrates a dramatic decrease in birth incidence of UVH patients most probably due to a corresponding increase in UVH TOP. Despite survival improved after introduction of Fontan surgery, survival has not improved significantly during the last 20years. [Copyright &y& Elsevier]

Abstract: Objectives: Study of outcomes of pregnancy in women with congenital heart disease. Material and methods: The cardiac state during and after pregnancy was analysed in 173 women (mean age 28 years, range 21–41) referred for fetal echocardiography and evaluation of maternal heart during 201 pregnancies. Acyanotic lesions were present in 152 women (100 operated), in 175 pregnancies; cyanotic lesions in 21, all operated, in 26 pregnancies. Eighteen patients in 20 pregnancies (9.9% pregnancies) presented an increased risk due to severe arrhythmias or relevant residual hemodynamic lesions (risk group). Results: Complications related to heart defects occurred in 9/201 (4.5%), pregnancies: in 6/175 (3.4%) pregnancies with acyanotic lesions, in 3/26 (11.5%) with cyanotic lesions and in 9/20 pregnancies of the risk group (45%), with a statistically significant difference with respect to the non-risk group. Two cases had serious tachyarrhythmias, five worsened hemodynamic state or cyanosis, one had hemorrhage post-partum on anticoagulants and one died of a rupture of isthmic aneurysm post-coarctectomy. Prematurity occurred in 19.2% of pregnancies with cyanotic and 5.7% with acyanotic lesions. There were 6/201 recurrences of cardiac anomaly (3%). Conclusions: Patients with uncomplicated heart disease had normal course of pregnancy, while complications due to serious arrhythmias or residual hemodynamical lesions and cyanosis occurred in 4.5% of pregnancies and, mainly in 45% of those with increased risk. Thorough evaluation of the cardiologic risk is mandatory for a correct counseling of women planning pregnancy. [Copyright &y& Elsevier]